[Primary amyloidosis AL as a cause of restrictive cardiomyopathy].
In a 71-year-old woman with macroglossia and heart failure, a monoclonal protein (kappa-light chains) was found in the serum. Congestive heart failure was diagnosed on the basis of heart catheterization and echocardiography. The tongue biopsy showed numerous amyloid fibrils, and plasmacytosis was present in the bone marrow. All these findings indicated a restrictive cardiomyopathy as part of a primary systemic amyloidosis.
Rückert
Y
Y
Universitätspoliklinik für Ambulante Spezialmedizin Charité, Humboldt-Universität zu Berlin.
Hansen
A
A
Dörffel
W V
WV
Eckert
B
B
Scholze
J
J
D000368:Aged; D000682:Amyloid / Q000097:blood*; D000686:Amyloidosis / Q000175:diagnosis* / Q000473:pathology; D001706:Biopsy; D002313:Cardiomyopathy, Restrictive / Q000175:diagnosis* / Q000473:pathology; D004452:Echocardiography; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood; D007145:Immunoglobulin kappa-Chains / Q000097:blood; D009206:Myocardium / Q000473:pathology; D013363:Submandibular Gland / Q000473:pathology; D014059:Tongue / Q000473:pathology
D000682:Amyloid; D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; C035222:amyloid protein AL
Primäre Amyloidose AL als Ursache der restriktiven Kardiomyopathie.